The price of life
One day, I may have to bury my son... Jeni Beviere, 42, Pembury
Arriving at Tunbridge Wells Hospital, I had a feeling of dread.
This can’t be good, I thought. It was May 2016, and my son Josh, 9, had been referred to hospital with suspected pneumonia.
For the last fortnight, he’d been withdrawn, constantly sleepy and had no appetite.
He didn’t even seem to have enough energy to play his video games.
At first, me and my husband Andy, 41, thought he had flu.
But it never let up.
And, after three trips to the GP, I began to worry when we still had no answers.
I felt sure that this wasn’t just flu. Something was wrong. I knew it in my gut.
My mum Helen, 72, took Josh to the GP for a fourth time, as I had to go to my sales job.
When she called to say she’d been told to take Josh to hospital, Andy and I raced to meet them.
The doctors ran tests, called us back a week later for the results.
And, when Andy and I were taken into a different room to Josh, I knew that it was bad news...
‘Josh has cystic fibrosis,’ the doctor revealed.
Josh had been born with the genetic disorder because both Andy and I carried the faulty gene.
It was a big shock. Usually diagnosed before the age of 1, the condition had caused a build up of mucus in Josh’s lungs and digestive system, and was preventing his body absorbing vital nutrients.
We were told that it was as if the trap door allowing mucus to drain from his lungs was locked shut.
It was a lifelong condition. But worse, it meant Josh might not live past 40. Distraught, I burst into tears, praying that I’d wake up from this nightmare. Only I didn’t. This was our new reality, and I had to be strong for Josh’s sake. It was a lot for him to take in at his age, and was extremely frightening for him. Plus, it’d completely change the way he lived. The biggest killer among CF sufferers is the slow decline in lung function. ‘So we have to keep your lungs as clear as possible,’ I explained.
If we didn’t do this, poor Josh would be prone to infections, bugs, viruses…
All of which would worsen his condition.
Josh had to take up to 40 tablets a day, and always needed to take medication before he ate.
Even if he was just sneaking a chocolate bar from the fridge!
Luckily, he understood how important it was, so he never slipped up.
Then, reading about record-breaking athlete Joshua Llewellyn-Jones, who also has CF, we realised the role exercise played in keeping Josh’s lungs clear.
Llewelyn-Jones had refused to give in to his condition, tackling extreme endurance challenges.
He credits exercise for keeping him alive.
Inspired by his story, Josh and Andy started cycling, swimming and running.
The cardio helped keep Josh’s lungs as healthy as
The condition meant Josh might not live past 40
possible. And, by staying active, he had more energy than he ever had before his diagnosis. But our boy faces a life-long battle. Last October, I joined a CF support group on Facebook.
There, I learned about an incredible new range of drugs from a pharmaceutical company called Vertex.
Current CF treatments deal with problems after they arise.
But Vertex has developed a precision medicine called Orkambi.
Orkambi is able to significantly slow the decline in lung function – the leading cause of death in CF sufferers.
Sadly, Orkambi isn’t compatible with Josh’s form of cystic fibrosis, but it can treat 50 per cent of CF sufferers in the UK.
‘This is amazing,’ I said to Andy, showing him what I’d found.
But it wasn’t all good news – Orkambi would cost a £100,000 a year per patient. Too expensive for the NHS. Vertex and the NHS had tried negotiating the price, but talks were at a standstill. I was stunned. Knowing what Josh went through on a daily basis, my heart sank for the thousands of CF patients with the CF mutation for which Orkambi could make a difference.
They knew there was a drug out there that could help them live longer.
‘But it’s just beyond their grasp!’ I gasped.
Tragic.
Since then, Vertex has begun trialling a triple combination treatment – using a new, experimental drug alongside a current double-drug treatment.
So far, it’s proved even more effective than Orkambi – and would be suitable for 90 per cent of CF sufferers. Including Josh!
‘It can open that trap door,’ I told Andy excitedly.
It could add decades to our boy’s life.
So, in January this year, I started actively campaigning for the introduction of the Vertex line of drugs into the NHS.
Together with hundreds of other patients and their loved ones, I’m pushing for the NHS to make another offer to Vertex.
Not just for Orkambi, but for access to the company’s whole line of medicines. Even those yet to come. At the moment, Josh is doing well. Exercising and a highcalorie,
high-protein diet has made him strong.
He’d always been a small boy, but he’s had quite the growth spurt!
But the possibility of burying my own son one day haunts me. It’s a shadow that follows me everywhere – and why I’m sharing his story.
I understand these medications cost a lot of money, but they could stop so much suffering and save so many lives – the lives of people like my son.
Can you really put a price on that?
It’s a shadow that follows me everywhere